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CASE MANAGEMENT, PRESENTATION, DISCUSSION AND SHARING OF INFORMATION ON EXTREMITY SARCOMAS by Michael Angelo L. Suñaz, M.D. Department of Surgery Ospital ng Maynila Medical Center CASE MANAGEMENT, PRESENTATION, DISCUSSION E.A., 63/M BINAN, LAGUNA CHIEF COMPLAINT: NON-HEALING WOUND ON THE RIGHT GLUTEAL AREA HISTORY OF PRESENT ILLNESS: 4 months PTA, the patient noted a pimple-like lesion on his right gluteal area. No other associated signs and symptoms were noted. HISTORY OF PRESENT ILLNESS: 3 ½ months PTA, the mass was noted to have increased in size. Consultation of a private physician was done and he was prescribed with unrecalled medications which afforded no relief. HISTORY OF PRESENT ILLNESS: 2 weeks PTA, the mass persisted and was now associated with occasional pain and undocumented fever. HISTORY OF PRESENT ILLNESS: Persistence of his condition prompted consultation and subsequent admission. PAST MEDICAL Hx: unremarkable FAMILY Hx: HPN - paternal side PERSONAL/SOCIAL Hx: - no history of smoking or alcoholic beverage intake PHYSICAL EXAMINATION: G/S: conscious, coherent, not in cardiorespiratory distress BP= 120/70 CR=83 RR= 19 T=38.20C SHEENT: no jaundice; pink palpebral cojunctiva,anicteric sclera, No NAD, No CLAD, No TPC PHYSICAL EXAMINATION: C/L: SCE, no retractions, clear BS CVS: adynamic precordium, NRRR, no murmur Abdomen: flat; soft; no palpable masses PHYSICAL EXAMINATION: Extremities: 10 x 13 cm firm, slightly movable, ulcerating mass, tender only upon deep palpation towards the right gluteal area SALIENT FEATURES: 63 y/o, M 10x13 cm firm, slightly movable, rapidly growing ulcerating mass tender only upon deep palpation towards the right gluteal area Occasional pain on the affected area Fever (38.20C) 10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating mass on the right gluteal area 10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating mass on the right gluteal area •Rapidly growing •With associated fever •Tenderness only upon deep palpation in the direction of the gluteal area 10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating mass on the right gluteal area Infectious •Rapidly growing •With associated fever •Tenderness only upon deep palpation in the direction of the gluteal area Neoplastic Clinical Diagnosis: Diagnosis Certainty Treatment Neoplastic Disease 75% Surgical Infectious Disease 25% Surgical/ Medical BASES: 63 y/o, M 10x13 cm firm, slightly movable, rapidly growing ulcerating mass tender only upon deep palpation towards the right gluteal area Occasional pain on the affected area Fever (38.20C) Do I need a para-clinical diagnostic procedure? YES Paraclinical Diagnostic Procedures Benefit Risk Cost Availability Biopsy Can provide a histopathologic diagnosis to determine the primary treatment of the lesion. Bleeding Pain + Readily available MRI Accurately delineates muscle groups and distinguishes between bone, vascular structures, and tumor. Sagittal and coronal views allow 3D evaluation of anatomical compartments.1 none ++++ Not readily available CT SCAN Provide detailed survey of the abdomen and pelvis and delineate adjacent organs and vascular structures.1 Radiation Exposure +++ Not readily available Paraclinical Diagnostic Procedures CT Scan of the Pelvis (9/29/08) – A mixed density mass with areas of necrosis is seen arising from the right gluteus maximus muscle infiltrating into the subcutaneous fat measuring about 14 x 12.25 x 9.26 (CC x W x AP). The mass displaces the anal opening to the left. Paraclinical Diagnostic Procedures CT Scan of the Pelvis (9/29/08) – There are no enlarged lymph nodes. – No osteolytic nor blastic changes seen. Osteophytes are noted along the iliac margins and vertebral endplates. – The included bowel loops, prostate and urinary bladder are unremarkable. Paraclinical Diagnostic Procedures CT Scan of the Pelvis (9/29/08) IMPRESSION: – Right gluteal mass, consider sarcoma. – Tissue correlation suggested. – Degenerative osseous changes, pelvis. 10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating mass on the right gluteal area Infectious •Rapidly growing •With associated fever •Tenderness only upon deep palpation in the direction of the gluteal area Neoplastic Sarcoma Paraclinical Diagnostic Procedures CXR (9/3/08) Both lungs are clear. The aorta is sclerotic. The heart is not enlarged. Diaphragm and sulci are intact. IMPRESSION: Atheromatous Aorta . Paraclinical Diagnostic Procedures Liver Ultrasound (9/3/08) The liver is not enlarged. The ducts are not dilated. The echo pattern is homogenous. No focal mass lesion is seen. IMPRESSION: Negative study. Paraclinical Diagnostic Procedures Histopathology result (8/15/08) Gross The specimen consists of several dark brown irregular soft and friable tissues, 4.0 cm in agrregate. The entire specimen is taken for study Paraclinical Diagnostic Procedures Histopathology result (8/15/08) Microscopic Microsections disclose loose aggregates of malignant round cells exhibiting marked hyperchromasia, anisoneuclosis and prominent nucleoli. These have marked eosinophilia and moderate polymorphism. Some tumor giant cells are seen. These are admixed with necrotic and inflammatory material. Paraclinical Diagnostic Procedures Histopathology result (8/15/08) MALIGNANT ROUND CELL TUMOR, fragments of, admixed with abscess material. 10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating mass on the right gluteal area Infectious •Rapidly growing •With associated fever •Tenderness only upon deep palpation in the direction of the gluteal area Neoplastic Sarcoma Malignant Round Cell Liposarcoma Pretreatment Diagnosis: Diagnosis Certainty Treatment Malignant Round Cell Liposarcoma 95% Surgical/ Neoadjuvant, Adjuvant Therapy Gluteal Abscess 5% Surgical/ Medical TREATMENT PRETREATMENT DIAGNOSIS: MALIGNANT ROUND CELL LIPOSARCOMA, RIGHT GLUTEAL AREA TREATMENT GOALS OF TREATMENT: – Curative extirpation of the tumor TREATMENT OPTIONS TREATMENT BENEFIT RISK COST AVAIL En bloc Surgical Resection Removal of the gross tumor. Primary treatment modality.2 Local recurrence if done with inadequate margins. Bleeding. May require contiguous organ resection.2 ++ Available Pre-operative Radiation Therapy Allows early multidisciplinary planning while the tumor is in place.1 Allows lower doses to be delivered to an undisturbed tissue bed that is better oxygenated.1 Difficulty with pathological assessment of margins and increased incidence of wound complications.1 ++++ Not readily available TREATMENT OPTIONS TREATMENT BENEFIT Pre-operative Size of the preRadiation Therapy operative radiation fields and the number of joints included in the field are significantly smaller which may result in an improved functional outcome.1 RISK COST AVAIL TREATMENT OPTIONS TREATMENT BENEFIT Post-operative Lower wound Radiation Therapy complication rate. RISK Larger radiation field. COST ++++ AVAIL Not readily avalable TREATMENT OPTIONS TREATMENT Brachytherapy BENEFIT RISK Less radiation scatter and much shorter duration of therapy.2 Indicated only in the setting of high-grade lesions.2 Rates of wound complications similar to those of postoperative external beam radiotherapy.2 COST ++++ AVAIL Not readily avalable TREATMENT OPTIONS TREATMENT Adjuvant systemic chemotherapy BENEFIT Statistically significant improvements in local recurrence, distal recurrence,and disease-free survival rates ranging from 6%-10%. 4% improvement in overall survival.2 RISK Potential toxicity.2 COST ++++ AVAIL Available TREATMENT OPTIONS TREATMENT Neoadjuvant systemic chemotherapy BENEFIT RISK Ability to assess Potential toxicity tumor responsiveness to the give chemotherapeutic agents, early treatment of metastatic disease, and downstaging of primary tumor.2 COST ++++ AVAIL Available TREATMENT OF CHOICE WIDE RESECTION AND POSTOPERATIVE RADIATION THERAPY PREOPERATIVE PREPARATION Informed consent Psychosocial support Optimize patient’s health Screen for any condition that will interfere with treatment Prepare materials OPERATIVE TECHNIQUE Patient supine under CLEA Asepsis/Antisepsis Sterile drapes placed Intraoperative findings noted: Mass noted to have extended partially to the serosal layer of the rectum and outermost layer of the sphincter muscle. Gluteus maximus muscle mass and sciatic nerve intact. OPERATIVE TECHNIQUE Wide excision with 1 cm margin; flap created. Hemostasis Placement of drain Correct sponge and instrument count Apposition of flap with silk 2-0 Dry sterile dressing OPERATION DONE: WIDE RESECTION OF RIGHT GLUTEAL MASS POST OPERATIVE DIAGNOSIS Malignant round cell tumor (liposarcoma), right gluteal area *Final histopathology report still pending SHARING OF INFORMATION SARCOMAS Refer to tumors that show evidence of mesenchymal differentiation. 1% of adult malignancies 15% of pediatric malignancies • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Account for nearly 50% of adult sarcomas. • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Most common types : – – Liposarcoma Malignant Fibrous Histiocytoma (MFH) • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Liposarcomas: – – – Well-differntiated Myxoid/ round-cell Pleomorphic • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Diagnosis – – – Comprehensive history and PE Mass is the most common presenting complaint Frequently, a trivial traumatic event draws attention to the area (although there is probably no causal relation between a history of trauma and the development of a sarcoma). • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Diagnosis – Core needle biopsy Typically performed as the first step Can diagnose the presence of a sarcoma and grade it in 80% of the cases. For histologic type, it has an accuracy of 75%. • Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 EXTREMITY SARCOMAS Staging – Primary Tumor (T) Tx – primary tumor cannot be assessed T0 – no evidence of primary tumor T1 – tumor is < or equal to 5 cm in its greatest dimension • • T1a – tumor is above the superficial fascia T1b – tumor invading or deep to the superficial fascia • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Staging – Primary Tumor (T) T1 – tumor is > 5 cm in its greatest dimension • • T2a – tumor is above the superficial fascia T2b – tumor invading or deep to the superficial fascia • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Regional Lymph Nodes (N) – Nx – regional lymph nodes cannot be assessed – N0 – no regional lymph node metastasis – N1 – Regional lymph node metastasis • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Distant Metastasis (M) – Mx – distant metastasis cannot be assessed – M0 – no distant mmetastasis – M1 – distant metastasis • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Histopahological Grade (G) – Gx – Grade cannot be assessed – G1 – well-differentiated – G2 – Moderately differentiated – G3 – poorly differentiated – G4 - undifferentiated • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Stage Grouping – Stage 1 A – G1-2, T1a-1b, N0, M0 B – G1-2, T2a, N0,M0 • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Stage Grouping – Stage II A – G1-2, T2b, N0, M0 B – G3-4, T1a-1b, N0,M0 C – G3-4, T2a, N0,M0 • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Stage Grouping – Stage III G3-4, T2b, N0,M0 • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 EXTREMITY SARCOMAS Stage Grouping – Stage IV Any G, Any T, N1, M0 Any G, Any T, N0, M1 • Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 TREATMENT OPTIONS TREATMENT BENEFIT RISK COST AVAIL En bloc Surgical Resection Removal of the gross tumor. Primary treatment modality.2 Local recurrence if done with inadequate margins. Bleeding. May require contiguous organ resection.2 ++ Available Pre-operative Radiation Therapy Allows early multidisciplinary planning while the tumor is in place.1 Allows lower doses to be delivered to an undisturbed tissue bed that is better oxygenated.1 Difficulty with pathological assessment of margins and increased incidence of wound complications.1 ++++ Not readily available TREATMENT OPTIONS TREATMENT BENEFIT Pre-operative Size of the preRadiation Therapy operative radiation fields and the number of joints included in the field are significantly smaller which may result in an improved functional outcome.1 RISK COST AVAIL TREATMENT OPTIONS TREATMENT BENEFIT Post-operative Lower wound Radiation Therapy complication rate. RISK Larger radiation field. COST ++++ AVAIL Not readily avalable TREATMENT OPTIONS TREATMENT Brachytherapy BENEFIT RISK Less radiation scatter and much shorter duration of therapy.2 Indicated only in the setting of high-grade lesions.2 Rates of wound complications similar to those of postoperative external beam radiotherapy.2 COST ++++ AVAIL Not readily avalable TREATMENT OPTIONS TREATMENT Adjuvant systemic chemotherapy BENEFIT Statistically significant improvements in local recurrence, distal recurrence,and disease-free survival rates ranging from 6%-10%. 4% improvement in overall survival.2 RISK Potential toxicity.2 COST ++++ AVAIL Available TREATMENT OPTIONS TREATMENT Neoadjuvant systemic chemotherapy BENEFIT RISK Ability to assess Potential toxicity tumor responsiveness to the give chemotherapeutic agents, early treatment of metastatic disease, and downstaging of primary tumor.2 COST ++++ AVAIL Available MCQ 1. Sarcomas comprise how much of adult malignancies? a. 1% b. 3% c. 15% d. 20% MCQ 1. Sarcomas comprise how much of adult malignancies? a. 1% b. 3% c. 15% d. 20% MCQ 2. Sarcomas comprise how much of pediatric malignancies? a. 1% b. 3% c. 15% d. 20% MCQ 2. Sarcomas comprise how much of pediatric malignancies? a. 1% b. 3% c. 15% d. 20% MCQ 3. Extremity sarcomas comprise how much of adult sarcomas? a. 10% b. 30% c. 50% d. 20% MCQ 3. Extremity sarcomas comprise how much of adult sarcomas? a. 10% b. 30% c. 50% d. 20% MCR A – 1, 2, and 3 are correct B – 1 and 3 are correct C – 2 and 4 are correct D – only 4 is correct E – none are correct MCR I. Which of the following represents stage I soft-tissue sarcoma? 1. G1-2, T1a-T1b, N0,M0 2. G1-2, T2b, N0, M0 3. G1-2, T2a, N0,M0 4. Any G, Any T, N1, M0 MCR I. Which of the following represents stage I soft-tissue sarcoma? 1. G1-2, T1a-T1b, N0,M0 2. G1-2, T2b, N0, M0 3. G1-2, T2a, N0,M0 4. Any G, Any T, N1, M0 MCR I. Which of the following represents stage III soft-tissue sarcoma? 1. G1-2, T1a-T1b, N0,M0 2. G1-2, T2b, N0, M0 3. G3-4, T2a, N0,M0 4. G3-4, T2b, N0, M0 MCR I. Which of the following represents stage III soft-tissue sarcoma? 1. G1-2, T1a-T1b, N0,M0 2. G1-2, T2b, N0, M0 3. G3-4, T2a, N0,M0 4. G3-4, T2b, N0, M0 THANK YOU!!! REFERENCES Delman KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125 Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105 JOURNAL CRITICAL APPRAISAL Spinal metastases from myxoid liposarcoma warrant screening with magnetic resonance imaging Joseph H. Schwab, MD 1, Patrick J. Boland, MD 1, Cristina Antonescu, MD 2, Mark H. Bilsky, MD 3, John H. Healey, MD 1 * 1Department of Surgery, Orthopedic Service, Memorial SloanKettering Cancer Center, New York, New York 2Department of Pathology, Memorial Sloan- Kettering Cancer Center, New York, New York 3Department of Surgery, Orthopedic and Neurosurgery Services, Memorial Sloan-Kettering Cancer Center and Medical College of Cornell University, New York, New York email: John H. Healey ([email protected]) *Correspondence to John H. Healey, Department of Surgery, Orthopedic Service, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Suite A342, New York, NY 10021 ABSTRACT Background: – Myxoid liposarcoma (MLS) has an unusual tendency for extrapulmonary metastasis, particularly to the spine and soft tissues. The objective of this study was to determine the prevalence of spinal metastasis, treatment outcomes, and optimal screening method for spinal metastasis in patients with MLS. ABSTRACT Methods: – Data from patients with had spinal metastases were obtained from the authors' institutional soft tissue sarcoma database. The accuracy with which positron emission tomography (PET) scans and bone scans identified metastatic lesions was compared with the accuracy of magnetic resonance imaging (MRI). Clinical response to treatment was based on pain, neurologic scores, and survivorship analysis. ABSTRACT Results: – There were 33 patients who developed spinal metastasis after a median 36 months of follow-up (range, from 7.5 months to 33 years). Known spinal metastases were detected by bone scans in 16% of patients and by PET scans in 14% of patients. ABSTRACT Results: – Patients who underwent surgery had high- grade spinal cord compression more often than patients who did not undergo surgery (72% vs 19%, respectively; P = .002). Pain and neurologic function were improved or maintained in all patients who received radiation alone (n = 8 patients) and in all but 1 patient who underwent surgery (n = 18 patients). The median overall survival was 51.4 months from the time of primary diagnosis and 21.9 months from the time of first metastasis. ABSTRACT Conclusions: – Bone scans and PET scan lack sufficient sensitivity to detect spinal metastasis from MLS. Treatment of metastasis is palliative, but local treatment can yield long-term disease control in select patients. Screening with whole-spine MRI may lead to the earlier detection of spinal metastasis. Cancer 2007. © 2007 American Cancer Society. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Primary Guides: Was the assignment of patients to treatments randomized? – NO. Data from patients with had spinal metastases were obtained from the authors' institutional soft tissue sarcoma database. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Primary Guides: Were all patients who entered the trial properly accounted for and attributed at its conclusion? YES. All 33 MLS patients with spinal metastasis were accounted for. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Primary Guides: Was followup complete? YES. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Primary Guides: Were patients analyzed in the groups to which they were randomized? YES. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Secondary Guides: Were patients, health workers, and study personnel "blind" to treatment? NO. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Secondary Guides: Were the groups similar at the start of the trial? YES. Appraisal Guide: THERAPY OR PREVENTION Are the results of the study valid? Secondary Guides: Aside from the experimental intervention, were the groups treated equally? YES.